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儿童髓母细胞瘤治疗后的长期神经内分泌后遗症。

Long-term neuro-endocrine sequelae after treatment for childhood medulloblastoma.

作者信息

Heikens J, Michiels E M, Behrendt H, Endert E, Bakker P J, Fliers E

机构信息

Department of Internal Medicine, Emma Kinderziekenhuis, Amsterdam, The Netherlands.

出版信息

Eur J Cancer. 1998 Sep;34(10):1592-7. doi: 10.1016/s0959-8049(98)00212-3.

Abstract

The occurrence of neuro-endocrine deficiencies following craniospinal irradiation for medulloblastoma is well known, but data concerning the spectrum and prevalence of endocrine abnormalities in adulthood are scarce. We studied endocrine function in 20 (median age 25 years) adult subjects, 8-25 years (median 16 years) after therapy. The radiation dose to the whole cranium and spinal axis was 35 +/- 2.6 Gray (mean +/- standard deviation) with a boost to the posterior fossa of 18 +/- 3.7 Gray. 13 subjects had received additional chemotherapy. In 15 of 20 (75%) subjects, endocrine abnormalities were observed. In 14 (70%), growth hormone (GH) secretion was impaired; 7 (35%) subjects had an absolute GH deficiency, while 7 (35%) showed subnormal responses to insulin-induced hypoglycaemia. In contrast, only 20% (4) of these subjects showed impairment of the hypothalamus-pituitary-thyroid (HPT) axis, while 15% (3) showed central impairment of hypothalamus-pituitary-gonadal (HPG) function. Central impairment of the HPG axis was associated with impaired GH secretion in all cases. Central adrenal insufficiency was not observed. Basal levels of prolactin were normal in all subjects. Young age at treatment was a determinant of GH deficiency in adulthood (P = 0.014). Neither post-treatment interval, nor the use of chemotherapy were determinants of central endocrine impairment in adulthood. In long-term survivors of medulloblastoma, GH deficiency has a high prevalence. In contrast, impairment of the HPG and HPT axis is less common, while central adrenal insufficiency was not observed.

摘要

髓母细胞瘤患者接受颅脊髓照射后出现神经内分泌缺陷是众所周知的,但关于成年期内分泌异常的范围和患病率的数据却很稀少。我们研究了20名成年受试者(中位年龄25岁)的内分泌功能,这些受试者在治疗后8 - 25年(中位16年)。全颅和脊髓轴的辐射剂量为35±2.6格雷(平均±标准差),后颅窝加量至18±3.7格雷。13名受试者接受了额外的化疗。在20名受试者中的15名(75%)观察到内分泌异常。在14名(70%)受试者中,生长激素(GH)分泌受损;7名(35%)受试者存在绝对GH缺乏,而7名(35%)对胰岛素诱导的低血糖反应低于正常水平。相比之下,这些受试者中只有20%(4名)显示下丘脑 - 垂体 - 甲状腺(HPT)轴受损,而15%(3名)显示下丘脑 - 垂体 - 性腺(HPG)功能中枢受损。在所有病例中,HPG轴的中枢受损与GH分泌受损相关。未观察到中枢性肾上腺功能不全。所有受试者的催乳素基础水平均正常。治疗时年龄较小是成年期GH缺乏的一个决定因素(P = 0.014)。治疗后的时间间隔和化疗的使用都不是成年期中枢内分泌受损的决定因素。在髓母细胞瘤的长期存活者中,GH缺乏的患病率很高。相比之下,HPG和HPT轴的受损较少见,且未观察到中枢性肾上腺功能不全。

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